Abstract
We present a case of a neuroendocrine carcinoma (NEC) of the liver. A 66-year-old Japanese woman was admitted to our hospital with epigastric pain. Computed tomography revealed an 89×76 mm low density mass occupying most of the right lobe the liver with occlusion of the right portal vein (VP3) and right and middle hepatic vein (Vv2). Under a preoperative diagnosis of intrahepatic cholangiocarcinoma, extended right lobe hepatectomy, common bile duct resection, lymph node dissection, and hepatico-jejunostomy were performed. The histological findings were consistent mainly with NEC cells and adenocarcinoma cells in the intrahepatic bile duct. Both components were immunohistochemically positive for synaptophysin. Systemic chemotherapy (gemcitabine) for metastasis to the lung, liver and bone was ineffective and the patient died 6 months after surgery. Several cases of NEC of the liver have been reported, but this is the first reported case of NEC of the liver coexistent with intrahepatic cholangiocarcinoma. This case suggested that intrahepatic cholangiocarcinomas associated with the histological nature of NEC of the liver.